Poorer Prognosis of Idiopathic Pleuroparenchymal Fibroelastosis Compared with Idiopathic Pulmonary Fibrosis in Advanced Stage
Table 6
Prognostic factors for the overall survival of IPPFE patients during the follow-up period.
Parameter
HR (95% CI)
value
Univariate Cox proportional hazards model
Age
0.967 (0.887–1.053)
0.436
Sex (F/M)
4.866 (1.422–16.650)
0.012
Clubbed finger (p/n)
4.032 (1.098–14.801)
0.036
Fine crackles (p/n)
1.609 (0.338–7.658)
0.550
APDT/TDT (%)
0.983 (0.892–1.084)
0.737
BMI
0.990 (0.813–1.206)
0.921
Smoking history (y/n)
3.014 (0.876–10.374)
0.080
Pack-years
1.024 (1.004–1.045)
0.020
History of pneumothorax
7.829 (1.256–48.45)
0.027
GAP index
1.675 (1.153–2.435)
0.007
GAP stages (II + III/I)
10.841 (2.258–52.048)
0.003
SpO2 (%)
1.141 (0.708–1.838)
0.588
FVC %pred (%)
0.946 (0.909–0.984)
0.006
FEV1/FVC (%)
0.998 (0.954–1.043)
0.912
TLC %pred (%)
0.883 (0.824–0.953)
0.001
RV %pred (%)
0.992 (0.961–1.025)
0.642
RV/TLC %pred (%)
1.001 (0.984–1.018)
0.903
DLco %pred (%)
0.866 (0.799–0.939)
<0.001
DLco/VA %pred (%)
0.895 (0.831–0.964)
0.003
PaCO2 (Torr)
1.086 (0.909–1.298)
0.361
A-aDO2 (Torr)
1.090 (1.018–1.167)
0.013
ANA (40≥/40<)
1.144 (0.334–3.923)
0.831
SP-A (ng/ml)
1.006 (0.983–1.031)
0.604
SP-D (ng/ml)
1.001 (0.998–1.004)
0.601
KL-6 (U/ml)
1.001 (1.000–1.002)
0.027
ΔFVC (L)
0.016 (0.001–0.433)
0.014
ΔTLC (L)
0.001 (0.000–0.113)
0.005
ΔRV (L)
0.018 (0.000–1.811)
0.088
ΔRV/TLC (%)
1.018 (0.838–1.236)
0.857
ΔDLco (ml/min/mmHg)
0.021 (0.000–50180)
0.607
Lower lobe involvement (definite UIP and possible UIP/NSIP)
2.199 (0.626–7.730)
0.219
Multivariate Cox proportional hazards model
GAP index
2.510 (1.245–5.059)
0.010
FVC %pred (%)
0.903 (0.830–0.982)
0.017
APDT = anteroposterior diameter of the thorax; TDT = transverse diameter of the thorax; BMI = body mass index; GAP = (gender (G), age (A), and two lung physiology variables (P) (FVC and DLco)); SpO2 = arterial oxygen saturation measured by pulse oximetry; FVC = forced vital capacity; FEV1 = forced expiratory volume in 1 s; TLC = total lung capacity; RV = residual volume; DLco = diffusing capacity of the lung for carbon monoxide; A-aDO2 = alveolar-arterial oxygen difference; ANA = antinuclear antibody; SP = surfactant protein; KL-6 = Krebs von den Lungen-6.
